Chiari malformation as a clinical entity has been described more than hundred years ago. The concepts regarding pathogenesis, clinical features and management options have not yet conclusively evolved. Considering that a variety of treatment methods are being adopted to treat Chiari malformation is suggestive of the fact that confusion still reigns supreme in the minds of treating clinicians. Over the years, the understanding of Chiari malformation has changed from a disease process to a natural protective phenomenon and the treatment from decompression to fixation.